short bowel syndrome wong wui bun tuen mun hospital
TRANSCRIPT
Short bowel syndrome
1. Overview
2. Pathophysiology
3. Intestinal rehabilitation program
4. Medical treatment
5. Operative treatment
6. Transplantation
1. Overview• Definition:• Malabsorptive state that is associated with extensive
resection of small bowel as well as a range of congenital conditions (American College of Surgeons)
• Heterogeneous group of patients• Spectrum of disease severity• Reduced survival • (2 year 86%, 5 year 75%)• Significant morbidity
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2. Pathophysiology• Fluid, electrolyte and nutritional deficiencies• Dysregulation of enteric hormone• Disturbance in bowel motility• Change in bowel flora• Catheter related complications• Intestinal failure related liver failure• Bone resorption, gallstone and renal stones
Effect of anatomy on pathophysiology
Jejuno-ileal anastomtosis
Jejunocolic anastomosis
End jejunostomy
Probability of PN dependence
LowIncrease if <35 cm
VariableHigh if <60-65 cm
VariableHigh if <115 cm
Pathophysiology Reduced CCK, SecretinReduced gastrin clearance
Loss of enterohepatic circulation of bile saltVitamin B12 deficiencyBacterial overgrowth
MalabsorptionNo SCFA productionReduced GLP-1, GLP-2 and PYY
SymptomClinical problem
Transient gastric hypersecretion and emptying
SteatorrhoeaCholeretic diarrhoeaFat malabsorptionCholestasis
High stomal outputNet fluid loss
Surgical considerations• Limit resection• Use of second look operation• Prevention of stoma/ early closure• Preservation of ileocaecal valve
• Post-operative care• Early establishment of central venous assess• Early involvement of multi-disciplinary team
3. Intestinal rehabilitation program• Multidisciplinary, protocolized care• Combination of enteric nutrition +/- hormonal stimulation• Workload hypothesis• Oral nutrition stimulate intestinal adaptation
Morphological:Epithelial hyperplasiaIncreased villi heightIncreased crypt depthRemodeling of bowel
Functional:Up-regulation of transport molecule and brush border activity http://surgery.med.umich.edu/pediatric/chirp/clinical/mm/pathophysiology.shtml
3. Intestinal rehabilitation prgram• Systemic review 2013 by Stanger et.al.• Historical control (n=103) vs IRP (n=130)
• Reduction in septic episodes (0.3 vs. 0.5 event/month; p=0.01)
• Increase in overall patient survival (22% to 42%)
• Weaning from PN (RR=1.05, 0.88-1.25, p=0.62)• Incidence of IFALD (RR=0.2, 0-17.25, p=0.48• Relative risk of liver transplantation (3.99, 0.75-21.3,
p=0.11).
Enterotrophic hormoneGrowth hormone GLP-2 analog (Teduglutide)
Short term use Long term use
• Increase energy absorption• Gain in body weight
• 20% reduction of parenteral support
• Improved SBS-QoL scores
Lack of good evidence Double blind RCT available
• Metabolic complications• Acromegaly
• Contraindicated in malignancy
• Colonoscopic surveillance• Immunogenic
Intestinal failure
• Predictors:• Bowel length <100 cm• End jejunostomy/ jejunocolic anastomosis• Reduced Citrulline level (<20umol/L correlated with PN
dependence)
Definition:Failure of intestine to adequately meet the body’s requirement for fluid, macronutrients and micronutrients
Long term parenteral nutrition required
Medical treatment• Bacterial overgrowth• Increase parenteral nutrition requirement• D- lactic acidosis, mucositis, worsen diarrhoea• More common if ileocaecal valve absent• Empirical treatment with antibiotics
Medical treatment• Symptomatic care• Control of bowel motility and secretions• Lomotil, Imodium• Atropine• Proton pump inhibitor
Autologus intestinal reconstruction• Indicated in intestinal failure with complications
• Intestinal tapering• Longitudinal intestinal lengthening and tailoring (LILT)• Serial transverse enteroplasty (STEP)• Antiperistaltic segment• Colonic interposition
Choice of procedure• Preservation of absorptive surface• Dilated segment has impaired
peristalsis• Technical difficulty• Feasibility of procedure:• Any bowel dilatation?• Any previous procedure?
• Problem with transit time?• Antiperistaltic segment
• Patient with a dilated bowel?• STEP/ LILT
Operative treatmentLILT STEP
Performed once Repeated procedure feasible
Double length Variable increase in length
Uniform dilatation Tailored diameter
Dissection of mesentery No interference with blood supply
Peritoneal contamination No contamination
PN Dependence 45% PN Dependence 31 – 57%
Transplant 18.6% Transplant 4.8 – 29%
Role of operation• Improve bowel autonomy• Decrease PN requirement• Decrease need for transplantation• Reverse liver disease up to 80%
• Complications:• Intestinal obstruction• Anastomotic leakage• Bowel ischaemia• Mortality ~ 10%
Transplantation• Intestinal transplant• Combined liver-intestinal transplant
• Indications: • 1. Presence of PN-associated liver disease• 2. Loss of central venous access• 3. Recurrent catheter-related sepsis or a single episode of
fungal sepsis• 4. Recurrent bouts of severe dehydration or metabolic
abnormalities• (US Centers for Medicare and Medicaid Services)
• ?Better catheter care• ?Improved parenteral nutrition• ?Quality of life• Early referral to specialist centre
Bring home message
1. Limit bowel resection
2. Early stoma closure
3. Intestinal rehabilitation program
4. STEP vs LILT
5. Considerations for transplant
Reference1. Modern treatment of short bowel syndrome. Jeppesen PB. Curr Opin Clin Nutr Metab Care. 2013 Sep;16(5):582-7. doi:
10.1097/MCO.0b013e328363bce4. Review.
2. Short bowel syndrome – surgical perspectives and outcomes. Nicola Smith, Rachel Harwood, Sarah Almond. Paediatrics and Child Health Volume 24, Issue 11, November 2014, Pages 513–518
3. Serial transverse enteroplasty (STEP) for patients with short bowel syndrome (SBS). American College of Surgeons.
4. Management of short bowel syndrome. Jason P. SulkowskixJason P. Sulkowski. Pathophysiology. February 2014. Volume 21, Issue 1, Pages 111–118
5. Surgical management of short bowel syndrome. Iyer KR1. JPEN J Parenter Enteral Nutr. 2014 May;38(1 Suppl):53S-59S. doi: 10.1177/0148607114529446. Epub 2014 Mar 25.
6. Long-term outcome of short bowel syndrome in adult and pediatric patients. Wasa M1, Takagi Y, Sando K, Harada T, Okada A. JPEN J Parenter Enteral Nutr. 1999 Sep-Oct;23(5 Suppl):S110-2.
7. Effect of growth hormone, glutamine, and enteral nutrition on intestinal adaptation in patients with short bowel syndrome. Guo M, Li Y, Li J. Turk J Gastroenterol. 2013;24(6):463-8.
8. Short bowel syndrome: highlights of patient management, quality of life, and survival. Kelly DG1, Tappenden KA, Winkler MF. JPEN J Parenter Enteral Nutr. 2014 May;38(4):427-37. doi: 10.1177/0148607113512678. Epub 2013 Nov 18.
9. Overview of short bowel syndrome: clinical features, pathophysiology, impact, and management. Storch KJ1. JPEN J Parenter Enteral Nutr. 2014 May;38(1 Suppl):5S-7S. doi: 10.1177/0148607114525805. Epub 2014 Mar 6.
10. Pathophysiology of short bowel syndrome: considerations of resected and residual anatomy. Tappenden KA1. JPEN J Parenter Enteral Nutr. 2014 May;38(1 Suppl):14S-22S. doi: 10.1177/0148607113520005. Epub 2014 Feb 5.
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Elemental diet• Peptamen• Vivomax
• Monosaccharides• Disaccharides• Medium chain fatty acid• Amino acids• Vitamins• Minerals
Parenteral nutrition• >50% carbohydrates• 30-40 % fat emulsion• Amino acids• Electrolytes
• Additives:
• Vitalipid: Vitamin A, D2, E, K1
• Soluvit: Vitamin C, Vitamin H, Vitamin Bs, folic acid• Addamel: trace elements
Options of venous access• Considerations:• Venous thrombosis rate per 1000 catheter day• Sepsis rate per 1000 catheter day• Reusability
• Tunneled central venous access• Peripheral inserted central catheter (PICC)
• Aseptic technique• 70% ethanol block• Heparin solution flush
Benefit of stoma closure• Recruit of distal bowel for adaptation• Resumption of enterohepatic circulation of bile salt• Production of short chain fatty acid (SCFA) in colon• Activation of L cell for enteric hormone production
TransplantationPublication Results
Pironi 2011 5 year survival:Not indicated for transplant: 87% (95%CI 83-91%)Indicated but not transplanted 84% (95%CI 74-94%)Indicated and transplanted 54% (95%CI 29-79%)
Mazariegos 2010 1 year in 1992Graft survival 69%Patient survival 52%
1 year in 2012Graft survival 85%Patient survival 75%
Ceulemans LJ 2015 1 yearGraft survival 62.8%Patient survival 71.1%
5 year: Graft survival 58.7%Patient survival 53.1%
Grant 2015 Patient survival: 76%, 56% and 43% at 1, 5 and 10 yearsRates of graft loss beyond 1 year have not improved